Aortic Valve Disease In Children Research Articles

Coronary artery dilation associated with bicuspid and unicuspid aortic valve disease in children: a series of 17 patients

Coronary artery dilation associated with bicuspid/unicuspid aortic valves is described in adults with limited data in children. We aimed to describe the clinical course of children with bicuspid/unicuspid aortic valves and coronary dilation including coronary Z-score changes over time, association of coronary changes with aortic valve anatomy/function, and complications. Institutional databases were searched for children ≤18 years with both bicuspid/unicuspid aortic valves and coronary dilation (1/2006-6/2021). Kawasaki disease and isolated supra-/subvalvar aortic stenosis were excluded. Statistics were descriptive with associations measured by Fisher's exact test and overlapping 83.7% confidence intervals. Of 17 children, bicuspid/unicuspid aortic valve was diagnosed at birth in 14 (82%). Median age at coronary dilation diagnosis was 6.4 years (range: 0-17.0). Aortic stenosis was present in 14 (82%) [2 (14%) moderate, 8 (57%) severe]; 10 (59%) had aortic regurgitation; 8 (47%) had aortic dilation. The right coronary was dilated in 15 (88%), left main in 6 (35%), and left anterior descending in 1 (6%) with no relationship between leaflet fusion pattern or severity of aortic regurgitation/stenosis on coronary Z-score. Follow-up evaluations were available for 11 (mean 9.3 years, range 1.1-14.8) with coronary Z-scores increasing in 9/11 (82%). Aspirin was used in 10 (59%). There were no deaths or coronary artery thrombosis. In children with bicuspid/unicuspid aortic valves and coronary dilation, the right coronary artery was most frequently involved. Coronary dilation was observed in early childhood and frequently progressed. Antiplatelet medication use was inconsistent, but no child died nor developed thrombosis.

Autograft Stabilization for the Ross Procedure in Children

Aortic valve disease in children presents a considerable surgical challenge. When repair is not feasible, the Ross procedure represents the best option for replacement as it allows growth and does not require anticoagulation. However, there is a risk of progressive autograft dilatation, and eventual failure of the autograft with severe regurgitation. Autograft stabilization has proven beneficial in adults but has not been widely adopted in children undergoing the Ross procedure. We present a series of techniques for autograft stabilization which can be used at different stages of development, from infants to fully grown adolescents. Aortic valve disease in children presents a considerable surgical challenge. When repair is not feasible, the Ross procedure represents the best option for replacement as it allows growth and does not require anticoagulation. However, there is a risk of progressive autograft dilatation, and eventual failure of the autograft with severe regurgitation. Autograft stabilization has proven beneficial in adults but has not been widely adopted in children undergoing the Ross procedure. We present a series of techniques for autograft stabilization which can be used at different stages of development, from infants to fully grown adolescents.

Multicentre comparative analysis of long-term outcomes after aortic valve replacement in children

ObjectiveThe ideal valve substitute for surgical intervention of congenital aortic valve disease in children remains unclear. Data on outcomes beyond 10–15 years after valve replacement are limited but important for...

The utility of aortic valve leaflet reconstruction techniques in children and young adults

ObjectivesThe treatment of aortic valve disease in children and adolescents requires an individualized approach to provide a long-term solution with optimal hemodynamic profile. The role of aortic leaflet reconstruction techniques is evolving. MethodsWe retrospectively reviewed the charts of 58 patients who underwent aortic valve tricuspidalization either by an Ozaki procedure (neo-tricuspidalization) or single leaflet reconstruction between 2015 and 2019. Immediate operative results as well as hospital and short-term outpatient follow-up data were evaluated. ResultsFifty-eight patients underwent leaflet reconstruction with 40 (69%) receiving a neo-tricuspidalization and 18 patients (31%) undergoing single leaflet reconstruction, using either a glutaraldehyde fixed autologous pericardium or tissue engineered bovine pericardium (CardioCel; Admedus, Queensland, Australia). The median age at the time of surgery was 14.8 years (interquartile range, 10.6-16.8 years). Twenty-three patients (40%) had isolated aortic regurgitation. The peak velocity across the aortic valve decreased from 3.4 ± 1.2 meters per second (m/s) preoperatively to 2.0 ± 0.4 m/s (P < .001) after surgery and remained stable (2.2 ± 0.7 m/s) during a median echocardiographic follow-up of 14.1 months (7.2-20.1 months) for the whole cohort. Freedom from reoperation or moderate and greater aortic regurgitation at 1, 2, and 3 years was 94.2% ± 3.3%, 85.0% ± 5.8%, and 79.0% ± 8.0%, respectively, with no difference between the neo-tricuspidalization and single leaflet reconstruction groups (P = .635). There were 6 late reoperations (10%) of which 3 were due to endocarditis. ConclusionsAortic leaflet reconstruction provides acceptable short-term hemodynamic outcomes and proves the utility of this technique as an adjunctive strategy for surgical treatment of aortic valve disease in children and young adults.

Outcome of 40 consecutive cases of modified Ross procedure using novel Dacron valved conduit.

The Ross procedure is an established option for aortic valve disease in children. Due to limited availability of pulmonary homograft, we devised a novel technique for right ventricular outflow tract (RVOT) reconstruction by preparing indigenous Dacron valved conduit. Forty consecutive cases of modified Ross procedure done at our center (2013-2018) were analyzed. Thirty-seven patients (95%) were followed up with median duration of 2.5 (0.08-5.5) years. Median age was 12 (5-39) years. Nineteen (47.5%) patients had rheumatic aortic valve disease, while 19 (47.5%) had congenital aortic valve disease. Aortic root replacement with pulmonary autograft was performed in all patients. Dacron conduit for RVOT reconstruction was used with on table sewn bileaflet valve using Dacron patch (n = 22), expanded polytetrafluoroethylene (ePTFE) membrane (n = 10), bioprosthetic valve (n = 4), and pericardium (n = 4). Additional surgical procedures included mitral valve repair (n = 10), septal myectomy (n = 2), ascending aorta replacement (n = 1), ruptured sinus of valsalva (RSOV) repair (n = 1), and ventricular septal defect (VSD) closure (n = 1). There was one in-hospital mortality while one late death occurred at 3.5years postoperatively. The neo-aortic valve regurgitation on echocardiographic evaluation at last follow-up was trivial (n = 28), mild (n = 7), and moderate (n = 2). Mild RVOT obstruction was present in 8 patients while 18 patients had mild pulmonary regurgitation. No patient required reintervention during follow-up. Our early results of modified Ross procedure are encouraging, however, long-term follow-up is required.

Current surgical strategies and techniques of aortic valve diseases in children.

While the long-term outcome of surgical aortic valvotomy (SAV) appears to be better than that of balloon aortic valvuloplasty (BAV) as the primary procedure of aortic valve stenosis, the surgical strategies and techniques of treating aortic valve disease in children in other situations remain controversial. Valve repair should be first considered while replacement is still unavoidable in some cases, and new repair techniques developed by innovative surgeons are gradually becoming adopted. Some complex repair procedures such as cusp extension, leaflet replacement/reconstruction have provided satisfactory outcomes. The Ozaki technique replaces aortic valve leaflets with glutaraldehyde-treated autologous pericardium instead of replacing the valve entirely. Special instruments have been developed to make the Ozaki technique more reproducible and standardized. Neonates and infants undergoing aortic valve replacement (AVR) are a high-risk group, where repair should be the primary consideration rather than replacement. Several systematic reviews reveal that all currently available aortic valve substitutes such as pulmonary autograft, mechanical prosthesis, homograft and bioprosthesis are associated with suboptimal results in children, but pulmonary autograft appeared to be superior with high freedom from reintervention and better hemodynamic performance. The strategy for treatment of aortic valve disease should be specifically analyzed based on the brief of being beneficial for children.

Mid-term Outcome of 100 Consecutive Ross Procedures: Excellent Survival, But Yet to Be a Cure.

The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients). Additional procedures included Ross-Konno procedure (14%), resection of subaortic stenosis/myectomy (11%) and reduction plasty of the ascending aorta (25%). One patient died within the first 30days (1%). Late deaths occurred in 4 patients (4%) 0.5-4.5years postoperatively: causes included pulmonary hypertension due to endocardial fibroelastosis (2), subarachnoid haemorrhage (1) and sudden cardiac death (1). Five-year survival was 93.6 (95% CI 88.1-99.1)%. Moderate or severe aortic (autograft) regurgitation needing reoperation occurred in 8 patients with a 5-year freedom from autograft reoperation of 98.5 (95.6-100)%. Five-year freedom from reintervention (surgery or catheter based) on the right ventricular outflow tract conduit was 91.5 (85.5-96.5)%. Univariate predictors of this reinterventions were smaller graft size (p = 0.03) and use of a ContegraR graft (p = 0.04). Ross procedure can be performed with low mortality and good survival in the long term. Most of the reinterventions are related to the neo-right ventricular outflow tract and may be partly attributed to the lack of growth. While the Ross Procedure remains an invaluable option for aortic valve disease in children, new solutions for the neo-pulmonary valve as well as for the less often occurring problems on the autograft are needed.

LONG TERM RESULTS OF PAEDIATRIC ROSS PROCEDURE: A NEW METHODOLOGY OF TIME-TO-EVENT DATA META-ANALYSIS

Ross procedure has been used for over 40 years in the surgical management of irreparable aortic valve disease in children. Little clinical long-term information is available regarding the durability of the autograft in the aortic position and the pulmonary conduit substitute. Moreover, there is no fully established methodology to perform time-to-event data meta-analysis. The aim of study was to develop a new methodology to perform such meta-analysis and therefore pool the long term results of paediatric Ross procedure.

Biomechanics drive histological wall remodeling of neoaortic root: A mathematical model to study the expression levels of ki 67, metalloprotease, and apoptosis transition.

The pulmonary artery autograft (PA) is the ideal substitute for aortic valve disease in children and young adult. However, it is harnessed by the issue of long-term dilation and regurgitation, often requiring surgery. PA implanted in aortic position during the growth phase in children undergoes a process of mechanical remodeling. We previously developed a semiresorbable armored prosthesis able to mechanically sustain the neoaorta preventing dilation and to gradually integrate with the PA wall inducing a progressive arterial-like tissue positive remodeling. We also described the mechanisms of growth, remodeling and stress shielding of the reinforced PA through a mathematical model. We sought to demonstrate the biological counterpart and the potential molecular mechanisms underlying this histological and mechanical remodeling. A specific mathematical model was developed to describe mechanical behavior of the PA. Mallory trichrome red staining and immunohistochemistry for MMP-9 were performed to elucidate extracellular matrix remodeling phenomena. Apoptosis and cell proliferation were determined by TUNEL assay and immunohistochemistry for Ki67, respectively. An histological remodeling phenomenon sustained by increased level of MMP-9, augmented cell proliferation and reduced apoptosis in the reinforced PA was demonstrated. The mathematical model predicted the biomechanical behavior subtended by the histological changes of the PA in these settings. Changes in metalloproteinases (MMP-9), cell proliferation and apoptosis are the main actors in the remodeling process occurring after transposition of the PA into systemic regimens. Use of semiresorbable reinforcements might induce a positive remodeling of the PA in the context of Ross operation. © 2016 Wiley Periodicals, Inc. J Biomed Mater Res Part A: 104A: 2785-2793, 2016.

Regression of left ventricular hypertrophy in children following the Ross procedure

Left ventricular hypertrophy (LVH) frequently accompanies the progression of aortic valve disease in children. The extent of LVH regression following surgical relief of aortic valve disease in children has not been clearly elucidated. We hypothesized that significant regression of LVH will occur in children following the Ross procedure. We examined LVH over time in children <18 years of age who underwent the Ross procedure. Left ventricular mass index (LVMI) and corresponding z scores were calculated based on height, age and gender. Left ventricular hypertrophy was defined as an LVMI of > 39 g/m(2.7) and a z score of >1.6. Twenty-five children underwent the Ross procedure. The left ventricular mass increased proportionally with the growth of the child from baseline to the latest follow-up at 7.3 ± 2.9 years (121.1 ± 81.5 vs 133.1 ± 79.8 g, P = 0.4). However, 96% (24/25) of children demonstrated LVMI regression from baseline. Mean LVMI decreased from 70.8 ± 31.2 to 41.8 ± 16.6 g/m(2.7) (P < 0.001). Similarly, LVMI z scores decreased from 2.2 ± 1.2 to 0.2 ± 1.9 (P < 0.001). Freedom from LVH was 83% at 10 years. Examination of LVMI and z scores over time demonstrated that the largest decrease occurred after the first year, with continued gradual decline over 10 years of follow-up. The Ross procedure is effective in reversing LVH in children with aortic valve disease.

Contemporary experience with surgical treatment of aortic valve disease in children

Surgical treatment of aortic valve (AoV) disease in childhood involves complex decisions particularly in very small patients. There is no consensus regarding the optimum surgical option. The objective of this review was to analyze a contemporary experience of AoV surgery in a large children's hospital. A retrospective review of children (aged ≤ 18 years) undergoing AoV repair or replacement from June 1995 to December 2011 was carried out. A total of 285 AoV operations (97 repairs, 188 replacements) were performed on 241 patients. Hospital survival for repair was 98% and for replacements was 97%. At follow-up of repairs, there were 16 (17%) reoperations and 3 (3%) late deaths. Follow-up of AoV replacements demonstrated 31 (16%) reoperations (homograft 27, autograft 3, mechanical 1) and 8 (4%) late deaths (homograft 5, autograft 2, mechanical 1). Freedom from reintervention or death (FRD) was found to be lower in repairs for infants (P = .048) and truncal valves (P < .05). For AoV replacements, infants and patients who had concomitant CHD or homografts (P < .0001) had lower FRD. Cox regression analysis for AoV replacements identified infants and homograft root replacements at a higher risk for death/reoperation. AoV repairs and replacements were generally found to be associated with low death and reoperation rates at long-term follow-up. Infants had a lower freedom from reintervention or death after either an AoV repair or replacement, although truncal valve repairs and AoV replacement in patients with concomitant CHD were associated with lower valve survival. Among the valve options, homograft root replacement had a higher risk of death/reoperation and lowest freedom from reintervention or death.

Outcomes of the infant Ross procedure for congenital aortic stenosis followed into adolescence

The Ross procedure is used to treat aortic valve disease in children. The advantages include autograft growth, long-term durability, and avoidance of anticoagulation. Long-term follow-up of the Ross procedure in infancy is limited. We sought to characterize the long-term outcomes of infants undergoing the Ross procedure. We performed a retrospective review of all patients who underwent a Ross operation at 18 months of age or younger at New-York Presbyterian and Cardiothoracic Center of Monaco from 1991 to 2010. The clinical, catheterization, and surgical records were reviewed. The most recent follow-up information, including echocardiogram and electrocardiogram, was obtained and analyzed. A total of 34 patients underwent a Ross procedure at a median age of 6 months (range, 4 days to 18.4 months). All had congenital aortic stenosis. All but 1 patient had undergone previous surgical or catheter-based interventions. The median follow-up was 10.6 years (range, 1.4-20.4 years). There were 4 early deaths and 1 late transplant. The freedom from right ventricular outflow tract reintervention was 85% at 5 years and 64% at 10years. The freedom from autograft reintervention was 95.5% at 10 years. In 20 subjects, late follow-up echocardiograms showed a significant difference between the mean early and late Z scores of the autograft annulus (0.8 vs 2.4, P=.03), sinus (0.8 vs 2.8, P=.002), and sinotubular junction (1.2 vs 2.7, P=.04). Mild or less aortic insufficiency occurred in 17 subjects. None had significant aortic stenosis. The long-term outcomes of the Ross procedure in infants and toddlers are favorable despite moderate dilatation of the autograft. Reintervention at the right ventricular outflow tract is common.

The Ross Operation in Children and Young Adults: A Fifteen-Year, Single-Institution Experience

The optimal operation for aortic valve disease in children and young adults remains controversial. The Ross operation offers avoidance of anticoagulation and the potential for growth but is technically demanding and creates double-valve disease. The goal of this study is to report our experience with the Ross operation and the need for reintervention at intermediate follow-up. A retrospective review of Ross operations in a single surgeon experience from 1992 to 2007 was conducted. All echocardiograms were reevaluated by a single cardiologist. The cohort included 54 patients with a mean age of 13.5 years (range 0.5 to 35 years). Pulmonary autograft implantation was accomplished using root replacement (n=43), root inclusion (n=9), and Dacron tube root replacement (n=2). Follow-up was available for 47 patients (87%) at a mean length of 6.4 years. There were no deaths. Kaplan-Meier estimates of freedom from explantation at 10 years were 100% for the autograft and 71% for the homograft. Autograft insufficiency at latest follow-up was trivial in 37 patients (82%), mild in 6 patients (13%), and moderate in 2 patients (4%). Reintervention for the homograft included balloon dilation in 3 children and conduit change in 5 children (all≤2 years old at initial operation). The Ross operation can be performed in children and adults with low mortality and can provide a durable result for the aortic valve with a low incidence of aortic insufficiency. The need for homograft replacement during follow-up in our series was primarily limited to children who were age 2 years or younger at initial operation.

Complex Aortic Valve Disease in Children

Complex Aortic Valve Disease in Children

Dez anos de experiência com a operação de Ross

To evaluate the 10-year outcomes of the Ross Operation, analyzing survival rate, incidence of reoperations, and late performance of pulmonary autografts and homografts in the reconstruction of the right ventricular outflow tract. Two hundred and twenty seven patients with a mean age of 29.1 +/- 11 years underwent Ross operation from May 1995 to February 2005. The most prevalent etiology was rheumatic disease in 61% of the cases. Autografts were implanted using the total root replacement technique in 202 cases, with intraluminal cylinder in 20, and in the subcoronary position in 5. The right ventricular outflow tract was conventionally reconstructed with cryopreserved homografts (n = 160), with proximal extension of the homograft with pericardium (n = 41), and with decellularized homografts (n = 26). The postoperative follow-up ranged from 1 to 118 months (mean = 45.5 months). Hospital mortality was 3.5%, and long-term survival was 96.9% at ten years. No episodes of thromboembolism and only two cases of endocarditis occurred. Eleven patients underwent reoperation because of problems related to the auto and/or homograft, progression of rheumatic mitral valve disease, and iatrogenic coronary insufficiency. After 10 years, 96.4% and 96.2% of the patients were free from reoperation in the autograft and homograft groups, respectively. No late autograft dilatation was observed. Reconstruction of the left ventricular outflow tract with decellularized homografts significantly reduced the incidence of gradients on late follow-up. Late outcomes with the Ross Operation were associated with an excellent long-term survival and a low incidence of reoperations and late morbidity. We consider this procedure the best option for the surgical treatment of aortic valve disease in children and young adults.

A Clinicopathological Study on Aortic Valves in Children

In order to investigate the clinicopathological characteristics of aortic valve disease in children, all the native surgically excised aortic valves obtained between January 2003 and December 2005 were studied macroscopically and microscopically. The patients’ medical records were reviewed and the clinical information was extracted. According to preoperative echocardiography, intraopera- tive assessment, and postoperative pathology, combined with clinical symptoms and signs, aortic valve diseases were divided into three categories: aortic stenosis (AS), aortic insufficiency (AI), and aortic stenosis with insufficiency (AS-AI). The etiology was determined according to the macro- scopic, microscopic and clinical findings. The results showed that among 70 aortic valves, patient age ranged from 6 to 18 years, with a mean of 15.4 years, and there were 56 boys and 14 girls (male: fe- male=4:1). Forty-four children only had pure aortic valve disease, and the other 26 children had aor- tic valve disease associated with other heart valve diseases. There were 5 cases of AS (7.14%), 60 cases of AI (85.71%) and 5 cases of AS-AI (7.14%). The causes were congenital aortic valve mal- formation (32 cases, 45.71%), rheumatic disease (28 cases, 40%), infective endocarditis (7 cases, 10%), Marfan syndrome (2 cases, 2.86%), and undetermined (1 case, 1.43%). It was concluded that the common causes of aortic valve disease in order of frequency in children were congenital aortic valve malformation, rheumatic disease, infective endocarditis, and Marfan syndrome. AI was more common in children with aortic valve disease. Compared with adult patients, congenital bicuspid aor- tic valve in children was often AI. Histologically, the leaflets of congenital bicuspid aortic valve were mainly myxomatous, fibrosis and calcification less seen. AI was frequently found in rheumatic dis- ease, mostly associated with other heart valve diseases. Macroscopic and microscopic examinations together with clinical information, echocardiographic findings a

The Ross procedure is the procedure of choice for congenital aortic valve disease

Objectives: The Ross procedure has emerged as an attractive option for aortic valve replacement in children and young adults. Our objective was to review our experience with the Ross procedure in young patients with congenital aortic valve disease. We also sought for evidence of growth in the autograft. Methods: From January 1990 to July 2000, 260 patients underwent the Ross procedure for various aortic valve diseases. There were 136 patients less than 18 years of age. Fifty-three (38%) of these patients had congenital aortic valve disease. Ages ranged from 3 months to 18 years (mean, 8 ± 5 years; median, 9 years). Ten patients were less than 2 years of age. Pure aortic stenosis was present in 18 patients, mixed stenosis and regurgitation in 32, and pure aortic regurgitation in 3. The aortic valve was bicuspid in 29 patients. Twenty-nine patients had previous procedures, mostly balloon dilation of the aortic valve (n = 8) or surgical aortic valvotomy (n = 12). Results: In all patients immediate results demonstrated a normally functioning neoaortic valve with not more than trivial aortic valve regurgitation. In the patients with stenosis, all levels of obstruction were relieved, and the gradient across the left ventricular outflow tract was completely abolished. Hospital mortality was 3 (5.6%) of 53 (overall Ross mortality was 34 of 260 [1.5%]). The patients were followed up for a mean of 4 years and up to 10 years. One patient died late of a noncardiac cause. Actuarial survival at 10 years was 94% ± 2%, and freedom from all events was 93% ± 5%. Only 1 patient needed autograft replacement for endocarditis. Intervention related to right ventricle-pulmonary artery conduit was required in 3 patients: balloon dilatation in 2, and reoperation in 1. At last follow-up, all patients but one were classified as being in New York Heart Association functional class I or II with normal or near-normal autograft valve function. Serial measurement of the left ventricular outflow tract and aortic root showed that as patients grew, the size of the outflow tract increased. When indexed to body surface area, this increase correlated with the patients' expected somatic growth. Conclusions: The Ross procedure for congenital aortic valve disease in children and young adults offers excellent hemodynamics, with the added advantage of real potential for growth. It should be considered the treatment of choice in this age group.J Thorac Cardiovasc Surg 2002;123:437-42

Early results of the Ross procedure in simple and complex left heart disease.

The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. Benefits include the lack of anticoagulation after surgery and the potential growth and durability of the autograft. The purpose of this study was to review our institutional experience with the Ross procedure and to compare early outcome in simple aortic valve disease and complex left heart disease. Between January 1995 and October 1998, 66 patients (median age, 10.8 years; range, 6 days to 34.8 years) underwent the Ross procedure. The primary indication for surgery was isolated valvular disease in 41 patients: aortic stenosis (AS; n=3), aortic insufficiency (AI; n=11), and AS/AI (n=27). The remaining 25 patients had multiple levels of left ventricular outflow tract obstruction, 12 of whom had at least moderate AI. Additional left heart disease in the complex group included subaortic stenosis (n=20), arch obstruction (n=7), mitral valve disease (n=5), apical aortic conduit stenosis or insufficiency (n=3), and supravalvar AS (n=2). There were 123 prior interventions performed in 51 patients, including aortic valvotomy/valvuloplasty (n=56), coarctation repair (n=21), subaortic stenosis resection/Konno procedure (n=10), ventricular septal defect closure (n=8), apical aortic conduit placement (n=3), aortic valve replacement (n=3), and other (n=22). An isolated Ross procedure was performed in 41 patients, 10 of whom required concurrent aortic annulus enlargement procedure to accommodate the larger pulmonary autograft. In the remaining 25 patients, 49 concurrent procedures were performed, including the Konno procedure (n=17), aortic annulus enlargement (n=2), subaortic membrane resection (n=9), arch augmentation (n=5), mitral valvuloplasty (n=5), ventricular septal defect closure (n=4), apicoaortic conduit division (n=3), and other (n=4). One patient (1.5%) died 3 days after a Ross-Konno procedure, which included arch reconstruction, from presumed arrhythmia. There were no other early deaths. One patient required ECMO (extracorporeal membrane oxygenation) for 3 days after a ventricular tachycardia (VT)-related cardiac arrest. Transient complete heart block was seen in 4 patients; the duration was <5 days. No patient had left ventricular outflow tract obstruction on discharge echocardiography. Neo-AI was graded as none (n=5), trivial-mild (n=57), or moderate (n=3). All 3 patients with moderate neo-AI at discharge had abnormal pulmonary valves before surgery. Perioperative VT was noted in 18 patients (27.2%), 2 of whom were discharged on antiarrhythmic medication. The Ross procedure can be performed in isolation or in combination with other complex procedures with low mortality (1.5%) and acceptable short-term results, even in patients with complex left heart disease and multiple prior interventions. Postoperative VT is common. Anatomic abnormalities of the pulmonary valve preclude its use as an autograft.

Early Results of the Ross Procedure in Simple and Complex Left Heart Disease

Background —The Ross procedure has been used increasingly to treat aortic valve disease in children and young adults. Benefits include the lack of anticoagulation after surgery and the potential growth and durability of the autograft. The purpose of this study was to review our institutional experience with the Ross procedure and to compare early outcome in simple aortic valve disease and complex left heart disease. Methods and Results —Between January 1995 and October 1998, 66 patients (median age, 10.8 years; range, 6 days to 34.8 years) underwent the Ross procedure. The primary indication for surgery was isolated valvular disease in 41 patients: aortic stenosis (AS; n=3), aortic insufficiency (AI; n=11), and AS/AI (n=27). The remaining 25 patients had multiple levels of left ventricular outflow tract obstruction, 12 of whom had at least moderate AI. Additional left heart disease in the complex group included subaortic stenosis (n=20), arch obstruction (n=7), mitral valve disease (n=5), apical aortic conduit stenosis or insufficiency (n=3), and supravalvar AS (n=2). There were 123 prior interventions performed in 51 patients, including aortic valvotomy/valvuloplasty (n=56), coarctation repair (n=21), subaortic stenosis resection/Konno procedure (n=10), ventricular septal defect closure (n=8), apical aortic conduit placement (n=3), aortic valve replacement (n=3), and other (n=22). An isolated Ross procedure was performed in 41 patients, 10 of whom required concurrent aortic annulus enlargement procedure to accommodate the larger pulmonary autograft. In the remaining 25 patients, 49 concurrent procedures were performed, including the Konno procedure (n=17), aortic annulus enlargement (n=2), subaortic membrane resection (n=9), arch augmentation (n=5), mitral valvuloplasty (n=5), ventricular septal defect closure (n=4), apicoaortic conduit division (n=3), and other (n=4). One patient (1.5%) died 3 days after a Ross-Konno procedure, which included arch reconstruction, from presumed arrhythmia. There were no other early deaths. One patient required ECMO (extracorporeal membrane oxygenation) for 3 days after a ventricular tachycardia (VT)–related cardiac arrest. Transient complete heart block was seen in 4 patients; the duration was &lt;5 days. No patient had left ventricular outflow tract obstruction on discharge echocardiography. Neo-AI was graded as none (n=5), trivial-mild (n=57), or moderate (n=3). All 3 patients with moderate neo-AI at discharge had abnormal pulmonary valves before surgery. Perioperative VT was noted in 18 patients (27.2%), 2 of whom were discharged on antiarrhythmic medication. Conclusions —The Ross procedure can be performed in isolation or in combination with other complex procedures with low mortality (1.5%) and acceptable short-term results, even in patients with complex left heart disease and multiple prior interventions. Postoperative VT is common. Anatomic abnormalities of the pulmonary valve preclude its use as an autograft.